title: Movement disorders in GLUT1 deficiency syndrome respond to the modified Atkins diet.
abstract: BACKGROUND
  Movement disorders are a prominent feature of glucose transporter-1 (GLUT1) deficiency syndrome (GLUT1DS). First-choice treatment is a ketogenic diet, but compliance is poor. We have investigated the effect of the modified Atkins diet as an alternative treatment for movement disorders in GLUT1DS.
  
  
  METHODS
  Four patients with GLUT1DS ages 15 to 30 years who had movement disorders as the most prominent feature were prospectively evaluated after initiation of the modified Atkins diet. Movement disorders included dystonia, ataxia, myoclonus, and spasticity, either continuous or paroxysmal, triggered by action or exercise. Duration of treatment ranged from 3 months to 16 months.
  
  
  RESULTS
  All patients reached mild to moderate ketosis and experienced remarkable improvement in the frequency and severity of paroxysmal movement disorders. Cognitive function also improved subjectively.
  
  
  CONCLUSIONS
  The modified Atkins diet is an effective and feasible alternative to the ketogenic diet for the treatment of GLUT1DS-related paroxysmal movement disorders in adolescence and adulthood.
authors.affiliation: Department of Neurology, Radboud University Medical Centre, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen, the Netherlands.
authors.forename: Wilhelmina G
authors.initials: WG
authors.lastname: Leen
authors.forename: Leena
authors.initials: L
authors.lastname: Mewasingh
authors.forename: Marcel M
authors.initials: MM
authors.lastname: Verbeek
authors.forename: Erik-Jan
authors.initials: EJ
authors.lastname: Kamsteeg
authors.forename: Bart P
authors.initials: BP
authors.lastname: van de Warrenburg
authors.forename: Michel A
authors.initials: MA
authors.lastname: Willemsen
mesh_terms: D000293:Adolescent; D000328:Adult; D001259:Ataxia / Q000178:diet therapy / Q000209:etiology; D002239:Carbohydrate Metabolism, Inborn Errors / Q000178:diet therapy* / Q000235:genetics; D003072:Cognition Disorders / Q000178:diet therapy / Q000209:etiology / Q000523:psychology; D050528:Diet, Carbohydrate-Restricted / Q000009:adverse effects; D005260:Female; D006801:Humans; D007662:Ketosis / Q000178:diet therapy / Q000209:etiology; D008297:Male; D009004:Monosaccharide Transport Proteins / Q000172:deficiency* / Q000235:genetics; D009069:Movement Disorders / Q000178:diet therapy* / Q000235:genetics; D009207:Myoclonus / Q000178:diet therapy / Q000209:etiology; D010349:Patient Compliance; D012640:Seizures / Q000209:etiology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D009004:Monosaccharide Transport Proteins
keywords: GLUT1 deficiency syndrome; ataxia; modified Atkins diet; myoclonus; paroxysmal exercise induced dyskinesia